Microscopic polyangiitis. Delineation of a cutaneous-limited variant associated with antimyeloperoxidase autoantibody

Irvine, Alan D.; Bruce, Ian N.; My, Walsh; Ea, Bingham

doi:10.1001/archderm.133.4.474

Cited by 16 publications

(22 citation statements)

References 18 publications

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“…11 A limited cutaneous variant of MPA has been reported, and like other forms of ANCA-associated vasculitis, p-ANCA titers correlated with disease activity. 169,170 At the other end of the spectrum, MPA, as a dynamic vasculitis, could represent an intermediate stage between limited vasculitis to systemic vasculitis with granulomatosis. 200 Up to 15% of MPA can first present with cutaneous disease 9 and 65% or less of all MPA patients have skin disease.…”

Section: Churg-strauss Vasculitismentioning

confidence: 99%

“…169,[201][202][203][204][205][206][207][208][209][210] The most common skin lesions found in MPA are (palpable) purpura and petechiae ($75%); other manifestations found in 20% or less of patients are splinter hemorrhages, nodules, palmar erythema, and livedo. 169,199,[201][202][203][204][205][206][207][208][209][210][211] Urticaria, hemorrhagic bullae, infarcts, facial edema, annular purpura, PG-like ulcers, EED, and telangiectases have been described in rare patients. 199,201,203,204,208,210 Most skin biopsies will demonstrate a small-vessel neutrophilic vasculitis that can affect dermal (upper and middermis mostly) or subcutaneous small vessels (,70%).…”

Section: Churg-strauss Vasculitismentioning

confidence: 99%

“…199,201,203,204,208,210 Most skin biopsies will demonstrate a small-vessel neutrophilic vasculitis that can affect dermal (upper and middermis mostly) or subcutaneous small vessels (,70%). 169,201,[203][204][205][206][207][208][209][210] Biopsies that fail to demonstrate small-vessel vasculitis show either a mixed lymphocytic and neutrophilic or predominately lymphocytic perivascular infiltrate affecting the upper and middermis 203,208 ; these findings may reflect the timing of the biopsy. An arteriolitis of deep dermal and subcutaneous vessels is found in a minority of biopsies and can be associated with panniculitis.…”

Section: Churg-strauss Vasculitismentioning

confidence: 99%

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Cutaneous Vasculitis Update: Small Vessel Neutrophilic Vasculitis Syndromes

Carlson

Kr²

2006

The American Journal of Dermatopathology

182

151

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A broad and diverse spectrum of vasculitic syndromes exists. These syndromes affect the skin with varying levels of associated systemic manifestations, running the gamut from a self-limited, localized, cutaneous phenomenon to rapidly progressive, multiorgan disease. The majority of cases of cutaneous vasculitis will show a neutrophilic small vessel vasculitis that can be either a primary (idiopathic) disorder (eg, cutaneous leukocytoclastic angiitis) or a secondary disorder that is associated with drugs, infection (eg, streptococcal infection, viral hepatitis), or underlying disease (eg, connective tissue disease, malignancy). Biopsy is the gold standard for the diagnosis of cutaneous vasculitis and also necessary for the detection of cutaneous vascular immune complexes by direct immunofluorescence. Based on the type of vessel disrupted by inflammation (small and/or muscular), the distribution of vasculitis in the dermis and subcutis, and predominate inflammatory cell-type mediating vessel wall damage, a list of relevant differential diagnoses can be generated. This histologic information coupled with extravascular findings such as tissue eosinophilia, tissue neutrophilia, and/or granulomas, plus pathophysiologic markers such as direct immunofluorescent examination for immune complexes and serologic evaluation for antineutrophil cytoplasmic antibodies allows for more accurate diagnosis of specific vasculitic entities. Herein, we review both primary and secondary vasculitic syndromes that affect the skin and show a small vessel neutrophilic mediated vasculitis.

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Section: Churg-strauss Vasculitismentioning

confidence: 99%

Section: Churg-strauss Vasculitismentioning

confidence: 99%

Section: Churg-strauss Vasculitismentioning

confidence: 99%

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Cutaneous Vasculitis Update: Small Vessel Neutrophilic Vasculitis Syndromes

Carlson

Kr²

2006

The American Journal of Dermatopathology

182

151

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“…7 The NLV subtypes Finkelstein's acute haemorrhagic edema of the skin, Henoch-Schoenlein purpura, microscopic polyarteritis nodosa or microscopic polyangiitis were considered in the differential diagnosis and were evaluated by internalists upon initial admission to the internal disease clinic. 9,10 However, dramatic progression of cutaneous necrosis and worsening of the general condition of the patient prompted us to consider purpura fulminans and necrotizing fasciitis as differential diagnoses.…”

Section: Discussionmentioning

confidence: 99%

Necrotizing leukocytoclastic vasculitis mimicking necrotizing fasciitis: a case report

et al. 2016

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<p>There are several subtypes of necrotizing leukocytoclastic vasculitis, which are classified according to their morphological features in biopsy specimens using immunofluorescence microscopy. Necrotizing leukocytoclastic vasculitis is limited to the skin, predominantly that of the lower extremities, and usually spares the palms and soles. The most common skin manifestation is palpable purpura. Other skin manifestations include maculopapular rash, bullae, papules, nodules, ulcers and livedo reticularis. There is no specific laboratory test to determine the diagnosis. There are various diseases presenting with these nonspecific symptoms, and a rapid differential diagnosis must be conducted, because the appropriate differentiation and diagnosis markedly influence the treatment strategy and survival of patients. In this study, we report a case of necrotizing leukocytoclastic vasculitis<strong> </strong>presenting with internal organ involvement and symptoms of necrotizing fasciitis, with emphasis on the clinical differentiation.</p>

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“…Other manifestations include splinter hemorrhages, nodules, palmar erythema, livedo, urticaria, hemorrhagic bullae, infarcts, facial edema, annular purpura, ulcers, and telangiectases (Carlson & Chen, 2006). One case of MPA reported in a patient with palpable purpura, myalgias, anorexia, and synovitis but no evidence of pulmonary or renal involvement suggests that a cutaneous limited variant of MPA may exist (Irvine et al, 1997). While a wide spectrum of pathologic changes can be seen, the classic histologic features of MPA on skin biopsy include a neutrophilic vasculitis of small vessels in the upper to middermis and subcutis (Carlson & Chen, 2006).…”

Section: Microscopic Polyangiitis (Microscopic Polyarteritis)mentioning

confidence: 99%